Polizotto et al reported criteria for the diagnosis of Kaposi sarcoma-associated herpesvirus (KSHV) inflammatory cytokine syndrome (KICS). The authors are from the National Cancer Institute (NCI).
Kaposi sarcoma-associated herpesvirus is the informal name for Human Herpesvirus 8 (HHV-8).
Patients with KICS have elevated serum levels of virus-associated IL-6 which is a more potent homolog of human IL-6 and IL-10. This triggers an inflammatory host response.
Parameters:
(1) clinical symptoms
(2) laboratory abnormalities
(3) radiographic abnormalities
(4) evidence of systemic inflammation (based on serum C-reactive protein concentration, CRP)
(5) evidence of KSHV lytic activity (based on KSHV viral load)
(6) exclusion of KSHV-associated multicentic Casteleman's disease
Working definition for the diagnosis of KICS:
(1) >= 2 findings in parameters 1 to 3 AND involve at least 2 of these 3 parameters
(2) presence of parameters 4, 5 and 6
Clinical symptoms:
(1) fever
(2) fatigue
(3) edema
(4) cachexia
(5) cough, dyspnea, airway reactivity and other respiratory symptoms
(6) nausea, anorexia, abdominal discomfort, altered bowel habits and other gastrointestinal symptoms
(7) arthralgias and myalgia
(8) altered mental status
(9) neuropathy with or without pain
Lymphadenopathy is not a prominent feature of KICS.
Laboratory abnormalities:
(1) anemia
(2) thrombocytopenia
(3) hypoalbuminemia
(4) hyponatremia
Radiographic abnormalities:
(1) lymphadenopathy
(2) splenomegaly
(3) hepatomegaly
(4) body cavity effusions
Evidence of systemic inflammation: serum CRP >= 3 g/dL (which seems incredibly high for a test with an upper limit of normal of < 1 mg/dL and which would rival the normal serum concentration of albumin)
Evidence of KSHV lytic activity: viral load in peripheral blood mononuclear cells >= 100 copies in 10^6 cells
Exclusion of multicentric Castleman's disease involves histopathologic evaluation of lymph node, bone marrow and/or spleen.
